Roshan Modi, MD Gregory Wrubel, MD Hartford Hospital Hartford, CT Concurrent thromboangiitis obliterans and moyamoya disease: a rare presentation with potential pathophysiological implications Roshan Modi, MD Gregory Wrubel, MD Hartford Hospital Hartford, CT

Background Thromboangiitis obliterans (Buerger’s disease) is a progressive vasculopathy of the distal extremities associated with tobacco use and thought to be immune mediated. Cerebral involvement in systematic disease is extremely rare and isolated cerebral disease (Spatz-Lindenberg disease) is an uncommon cause of vascular dementia. Currently, there is only one published report of moyamoya disease associated with thromboangiitis obliterans.

introduction Thromboangiitis obliterans (Buerger’s disease) is an inflammatory disorder that affects small and medium-sized arterial vasculature primarily in young, male smokers. Extremity vessels are most commonly affected with a characteristic “corkscrew” or “tree roots” appearance on angiography. Smoking is the primary risk factor and acts synergistically with other factors that predispose to vascular inflammation. The diagnosis is made clinically since there are no serologic markers to screen for.

introduction The steno-occlusive disease in moyamoya results in collateral circulation that resembles a “puff of smoke” (moyamoya's translation in Japanese) during angiography. Secondary complications of moyamoya include cerebral ischemia and intracranial hemorrhage. Patients can also be asymptomatic. The pathogenesis of moyamoya is unknown.

introduction Treatment for moyamoya includes the EDAS (encephaloduroarteriosynangiosis) procedure in which a scalp artery is dissected and a small opening is made in the skull beneath the artery so that it can be sutured to a branch of the middle cerebral artery to improve perfusion. The following case report demonstrates a rare occurrence of a patient with both Buerger’s disease and Moyamoya. No treatment other than smoking cessation has been found to stop disease progression. In severe cases, bypass grafts have been performed to salvage limbs.

Clinical presentation A 54-year old white male with a known history of Buerger’s disease presented with several days of headaches and dizziness and was found to have a subacute left PICA infarct. Work-up demonstrated a distal PICA occlusion thought to be embolic from a left subclavian atheroma proximal to the vertebral artery origin.

Angiography revealed a moyamoya pattern with occlusion of the right ICA terminus, severe steno-occlusive disease of the proximal right anterior and middle cerebral arteries, and robust lenticulostriate collaterals.

Clinical presentation A cerebral SPECT perfusion study revealed relative hypoperfusion throughout the right cerebral hemisphere that progressed following the administration of acetazolamide. An encephaloduroarteriosynangiosis was then performed.

discussion To prevent ischemia and/or intracranial hemorrhage, varying surgical procedures can be performed for direct and indirect revascularization. The direct revascularization technique involves intracranial mobilization of a branch of the superficial temporal artery (STA) and forming a direct arterial anastomosis between the mobilized STA branch and the ipsilateral middle cerebral artery (MCA) along the superficial surface of the brain. This procedure is known as the STA-MCA bypass and results in over 95% graft patency with excellent long term outcomes and is the first line recommendation. The indirect revascularization procedures include EDAS and EMS (encephalo-myo- synangiosis). In the EDAS procedure, a branch of the superficial temporal artery is placed onto the surface of the brain without forming a direct arterial anastomosis.

discussion Pandey and Steinberg have found that the surgical morbidity and mortality of STA-MCA revascularization was 3.5% and 0.7% respectively per treated hemisphere. The cumulative 5-year risk of perioperative or subsequent stroke or death was 5.5% and 98.1% of patients originally presenting with a TIA were without a recurrent TIA. They also found that morbidity and mortality of the indirect revascularization techniques were 0% and 5.7% respectively. The annual risk of cerebrovascular events was 0% in pediatric patients and 0.4% in adults in these patients. Angiography and stenting have also been proposed as treatment options for moyamoya however revascularization remains the preferred treatment due to reported failures.

discussion There has been only one published report of thromboangiitis obliterans with an associated moyamoya disease. Neurologic signs are very rare with thromboangiitis obliterans and occur in fewer than 2% of cases with the cortical surface and nearby territories being most often affected. Moyamoya is thought to be hereditary and afflicted individuals have also been found to have increased levels of thyroid antibodies. This uncommon presentation may have deeper pathophysiological meaning as possibly one autoimmune-mediated disease may increase the likelihood of the other. Other possibilities include moyamoya disease being a sequela of long standing thromboangiitis obliterans.

conclusion The association between moyamoya and Buerger's disease is rare and STA-MCA revascularization remains the first line treatment for moyamoya in this setting. Moyamoya associated with Buerger’s disease is a rare occurrence but treatment should be unchanged with STA-MCA revascularization as the first line recommendation.

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